The array of paediatric pulmonary complications of the various rheumatologic disorders illustrates both the complexities and challenges of the underlying disorders and the continuing lack of detailed knowledge of the pathophysiology and optimal treatment paradigms in children. While the vertical transfer of information has made much progress from adult studies, such as with the diagnosis and management of pulmonary arterial hypertension, in many instances underlying disorders may differ between children and adults in important and fundamental respects. Recognition of pulmonary complications of rheumatic disorders in children is often more difficult and requires anticipation and a high index of suspicion. Further progress in understanding and treating the various paediatric disorders is hampered by the lack of paediatric-specific information. Crucial to further progress are the expansion of orphan childhood disease databases and research networks. In this way a comprehensive approach to determining basic natural history, risks and outcomes, and defining the next generation of therapies in a disease-specific and age-specific manner can be achieved.
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